Die Diagnose der ANCA-assoziierten Vaskulitiden (AAV) setzt sich mosaikartig aus dem klinischen Befund, technischen Untersuchungsbefunden und der charakteristischen Histologie zusammen. Die Diagnostik sollte sich an dem klinischen „Leitsymptom” orientieren. Dabei hat sich für die weitergehenden Untersuchungen ein interdisziplinäres Vorgehen mit den unterschiedlichen Fachrichtungen bewährt. Bei der Auswahl der Therapie wird zwischen einer Remissionsinduktion und -erhaltung unterschieden. Cyclophosphamid gilt dabei als Standard-Therapie zur Remissionsinduktion, während sich Azathioprin, MTX und Leflunomid generell zur Erhaltung der Remission eignen. Die Etablierung einer stadienadaptierten Therapie hat wesentlich zur Verbesserung der Langzeitprognose von Patienten mit AAV geführt.
Summary
The diagnosis of ANCA-associated vasculitides (AAV) consists of clinical symptoms, results of technical procedures and a characteristical histology. The diagnostic workup should focus on the prominent clinical symptoms and should involve an interdisciplinary team of specialists. The therapy is divided into induction and maintenance treatment. Cyclophosphamid remains standard therapy for the induction of remission, whereas azathioprine, methotrexate and leflunomide are often used for maintenance therapy. The introduction of a stage-adapted treatment has contributed to a significant improvement of the long-time prognosis of patients with AAV.
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